ABSTRACT
Foix Chavany Marie syndrome, also called as bilateral opercular syndrome (OPS) was first described in 1837 by Magnus and further defined by Foix, Chavany and Marie in 1926 after whom it was named so. Here, we present a case of 37-year-old female, with known recurrent CVA who presented with sudden onset aphasia, dysphagia and difficulty in opening and closing her mouth and drooling of saliva. On imaging, patient was found to have ischemia of bilateral operculum.
ABSTRACT
Objective To summarize the clinical characteristics of Foix-Chavany-Marie syndrome.Methods The clinical,electroencephalography and imaging characteristics,as well as etiology,treatment and prognosis of 4 cases of Foix-Chavany-Marie syndrome were retrospectively evaluated,and relevant literature was reviewed to investigate its pathogenesis.Results All the 4 cases presented with hypersalivation,facial diplegia,dysarthria and dysphagia with autonomic-voluntary dissociation.Brain MRI showed bilateral cortical or subcortical lesions in the opercula and perisylvian areas.Three cases were secondary to encephalitis,and 1 case was related to perinatal hypoxic-ischemic encephalopathy.After administration of antiepileptic drugs,they were free of seizures.But all the other symptoms remained.Conclusions Autonomic-voluntary dissociation is a feature of Foix-Chavany-Marie syndrome.Most patients are due to bilateral structural or functional lesions between motor cortices and brain-stem cranial motor nuclei.The prognosis depends on the heterogeneous etiologies,such as encephalitis and cerebrovascular disease.
ABSTRACT
Foix-Chavany-Marie Syndrome (FCMS) is characterized by anarthria and bilateral facio-pharyngo-glosso-masticatory paralysis with an automatic-voluntary dissociation, which usually develops in bilateral opercular lesions. We present a case of FCMS caused by unilateral subcortical lesion. A 54-year-old man was admitted due to acute right hemiparesis with anarthria. He had voluntary facial paresis but automatic-involuntary facial movements were preserved. MRI showed an acute left corona radiata infarction and PET revealed decreased glucose metabolism in left basal ganglia and fronto-parietal lobe.
Subject(s)
Humans , Middle Aged , Basal Ganglia , Cerebral Infarction , Cranial Nerve Diseases , Cranial Nerves , Dissociative Disorders , Facial Paralysis , Glucose , Infarction , Paralysis , ParesisABSTRACT
Bilateral opercular syndrome or Foix-Chavany-Marie syndrome (FCMS) is characterized by facio-pharyngo-glosso-masticatory diplegia with an automatic-voluntary movement dissociation, which is usually caused by bilateral fron-toparietal opercular lesions. A 52 year-old man suddenly developed left hemiplegia and also presented with anarthria, dysphagia, difficulty in jaw opening and mastication. However, involuntary swallowing and slight control of jaw move-ments were partly preserved. His gag reflex was decreased and emotional incontinence was absent. Brain magnetic res-onance (MR) imaging revealed high signal lesions in the right middle cerebral artery territory and left anterior opercu-lum. Severe stenosis of the right middle cerebral artery was observed on a MR angiogram. Rehabilitation training by cueing has improved his ability to open the mouth. To our knowledge, this is the first report of FCMS in Korea, and a cautious differential diagnosis of pseudobulbar palsy or buccofacial apraxia may be crucial.